Pineal Region Tumors PDF

PINEAL REGION TUMORS
DIAGNOSIS & SURGICAL

APPROACHES
MODERATORS : DR.
DR ASHISH SURI
DR. SUMIT SINHA
PRESENTED BY : DR. UTKARSH BHAGAT
28 February 2011
PINEAL REGION
28 February 2011
PINEAL REGION
28 February 2011
ARTERIAL SUPPLY
PCA
P1
Quadrigeminal a.
a superior colliculi
P2
M P.ch
P ch
Pineal body, corpora quadrigemina, tela choroidea ventriculi
tertii, thalamus
L Pch
P h
Choroid plexus of lat. Ventricle, LGB, thalamus
28 February 2011
ARTERIAL SUPPLY
P3/P4
Medial occipital artery
Calcarine artery calcarine sulcus
Parietooccipital artery parietooccipital sulcus
Posterior pericallosal artery
SCA
Inferior colliculi
28 February 2011
VEINS OF PINEAL REGION

Great cerebral vein of Galen
Pineal veins(sup & inf) pineal body, trigonum
habenulae
Quadrigeminal veins(sup&inf)corpora
quadrigemina
Superior vermian vein superior vermis
Precentral cerebellar vein cerebellum, superior
cerebellar peduncle
Posterior pericallosal vein
Internal occipital vein
28 February 2011

Internal cerebral veins
Septal veins(ant&post) septum pellluvidum
Anterior caudate vein caudate nucleus
Thalamostriate(terminal) vein
Choroidal
Ch id l vein
i choroid
h id plexus
l
llat.
t V
Ventricle
ti l
Medial atrial (trigonal)vein Trigone
Direct
Di
llaterall veins
i
28 February 2011

Basal vein of Rosenthal
Vena cerebri media profunda
Venae centrales (striate) inferiores
Vena cerebri anterior
Vena
V
apicis
i i cornua temporalis
t
li (hippocampal
(hi
l vein)
i )
Vena atrii lateralis (lateral atrial vein )
Vena
V
cornus temporalis
li (atriotemporal
( i
l vein
i )
28 February 2011
DIFFERENTIAL DIAGNOSIS
GERM CELL TUMORS (account for 50%)
Pure germinoma
Embryonal cell carcinoma
Endodermal sinus tumor
Teratoma
T t
Mixed germ cell tumor
Choriocarcinoma
Ch i
i
28 February 2011
PINEAL PARENCHYMAL TUMORS
Pineoblastoma
Pineocytoma
TUMORS OF SUPPORTIVE TISSUES & ADJ. STR.

Astrocytoma
Ependymoma
Meningioma
Hemangiopericytoma
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10
Ganglioglioma
Ganglioneuroma
Chemodectoma
Quadrigeminal cistern lipoma
Metastatic tumors (very rare)

Lung, breast, stomach, kidney
Nonneoplastic lesions
Pineal cysts
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11
Arachnoid cysts
Cysticercous cysts
Vascular lesions
Aneurysmal dilatation of vein of Galen
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12
COMMON PATHOLOGIES
Germ cell tumors
Germinoma
Teratoma
Pineal cell tumors

Pineoblastoma
Pineocytoma
Other cell tumors & tumor like masses

Pineal cysts
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13
COMMON PATHOLOGIES
Astrocytoma
y
( thalamus,, midbrain,, tectum,, corpus
p
callosum)
Meningioma
Metastases
Vascular malformation ( Vein of Galen)
Misc.
Lipoma
Epidermoid
Arachnoid cyst
28 February 2011
14
EPIDEMIOLOGY
Incidence : <1% of CNS tumors

Germ cell tumors: more in pediatric pop., M>F
Pineal
i
l cellll tumors : more iin young adults,M=F
d l
Heritability: rare : E.g. Trilateral
retinoblastoma[ Pinealoblastoma + b/l
retinoblastoma, gene deletions ]
28 February 2011
15
Relative % of histological subtypes of I/C GCT

b
d on 399 tumors reviewed
i
db
i
based
by JJennings
et all
Embryonal ca.
4%
Endodermal
sinus
5%
Intrcranial GCT Chorioca.

4%
Mixed cell
16%
Germinoma
52%
Teratoma
19%
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16
Relative % of histological subtypes of pineal cell tumors based

on 90 tumors reviewed by Herrick & Rubinstein et al
Pineal cell tumors
Pineocytoma 42%
Pineoblastoma 32%
Mixed cell
28 February 2011
17
Most common age of onset of I/C GCT

reviewed
db
by Jennings et all
Germ cell
Ge
ce tu
tumors
os
18
16
14
12
10
8
6
4
2
0
28 February 2011
Germ cell tumors
18
Average age of onset for pineal cell tumors based on

90 tumors
t
reviewed
i
db
by H
Herrick
i k&R
Rubinstein
bi t i ett all
Pineal
ea cell
ce tu
tumors
os
40
35
30
25
20
Pineal cell tumors
15
10
5
0
Pineocytoma
28 February 2011
Mixed pineal
Pineoblastoma
19
CLINICAL FEATURES
Mechanism
Raised ICP [Hydrocephalus]
Direct cerebellar/brainstem compression
Endocrine dysfunction
Presentation
P
t ti
HEADACHE (most common) Aqueductal
obstruction
b t ti HCP
HCP
N/V, Papilledema, Obtundation, Cognitive deficits.
28 February 2011
20
CLINICAL FEATURES
EXTRAOCULAR PALSIES
PERINAUD SYNDROME
Paralysis of up gaze/convergence
Retractory nystagmus
Lightnear
g
p p
pupillary
y dissociation
4th CN PALSY
Diplopia
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21
CLINICAL FEATURES
Compression/infiltration of dorsal midbrain
Paralysis of down gaze
Ptosis
Inv. Of superior cerebellar peduncle

Ataxia/dysmetria
/
Inferior colliculi compression

Hearing disturbances
28 February 2011
22
CLINICAL FEATURES
Endocrine dysfunction: hypothalamic
invasion/HCP
Diabetes Insipidus
Precocious puberty[boys] : b h CG secrn. In
chorioca /Germinoma with STGC androgen
chorioca./Germinoma
secretion by Leydig cells
Pineal apoplexy
In vascular tumors : Pineal cell tumors/Chorioca.
28 February 2011
23
CSF Levels of tumor markers in germ

cellll tumors
Germino
G
i
ma
IImmatur
t
E b
Embryon
e
al cell ca
teratoma
Mature
M
t
Chorioca.
Ch
i
E d d
Endoderma
teratoma
l sinus
tumor
Alpha
Al
h
fetoprote
in
/+
/
+++
BetaHC
G
/+
/
++
+++
PLAP
++
28 February 2011
24
Other markers
C kit proto
Ckit
protooncogene
oncogene
CEA
LDH
B5 monoclonal antibody
S antigen, melatonin
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25
IMAGING
CT/MRI/ANGIOGRAPHY
Size of tumor: lateral & superior extent
Vascularity
Homogenous/heterogeneous
Irregularities
I
l iti off margination
i ti & probability
b bilit off
invasion
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26
IMAGING
ANATOMICAL relationships
28 February 2011
Inv of 3rd ventr/ position within 3rd ventr.

Extension into/above corpus callosum
Superolateral extension into ventricular trigone
Inv/compression of quadrigeminal cistern/aqueduct
Relation to ant. Cerebellar vermis
Location of deep venous system
27
GERMINOMA
T1W MRI : Isointense tumor obstructing

the
h aqueduct
d
off Sylvius
S l i
28 February 2011
28
GERMINOMA
33 year old female with visual loss, amenorrhea & DI
MRI shows germinomatous invasion of pineal
gland, optic stalk & floor of 3rd ventricle
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29
PINEOCYTOMA
Homogenously enhancing tumor with

cystic
i portion
i
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30
PINEOCYTOMA
Pineal apoplexy
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31
PINEOCYTOMA
Benign well differentiated cells forming

rosettes
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32
PINEOBLASTOMA
Highly cellular poorly differentiated cells

forming patternless sheets
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33
Mixed germ cell tumor

Multiple components : Endodermal
sinus embryonal cell,
sinus,
cell mature & immature
teratoma as revealed on biopsy following resection
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34
MATURE TERATOMA
28 February 2011
Nonkeratinizing squamous epithelium

alternating
l
i with
i h ciliated
ili d columnar
l
epithelium
35
MATURE TERATOMA
Periosteal tissue with mesenchymal

stroma
28 February 2011
36
MATURE TERATOMA
Cartilaginous tissue
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37
IMMATURE TERATOMA
Highly cellular primitive elements

resembling
bli ffetall neurall tube
b
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38
ENDODERMAL SINUS TUMOR
Schiller Duval body

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39
ADJUVANT THERAPY
RADIOTHERAPY
For malignant germ cell/pineal cell tumors: 4000cGy :
Ventricular system
y
1500cGy : Tumor bed
180 cGyy dailyy fractions

3500 cGy to spine if spinal seeding
radiographically documented.
RT maybe withheld: for histologically benign
pineocytoma /ependymoma completely resected.
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40
ADJUVANT THERAPY
Germinoma with raised b
b hCG has less
favorable prognosis.
Side effects of RT
Cognitive deficits.
Hypothalamic/Endocrine
H
h l i /E d i d
dysfunction.
f
i
Cerebral necrosis.
De novo tumor formation.
28 February 2011
41
ADJUVANT THERAPY
CHEMOTHERAPY
Indications
Non germinomatous malignant germ cell tumors
Germinoma with syncytiotrophoblastic giant cells
Recurrent //disseminated p
pineal cell tumors
Cisplatin/carboplatin + Etoposide
O
Others:
e s vincristine/lomustine/cyclophosphamide
c s e/ o us e/cyc op osp a de
28 February 2011
42
RADIOSURGERY
For tumors less than 3 cm

For tumors that recur locally.
Risk
i k off recurrence : pineal
i
l cell/germ
ll/
cellll tx.
No therapeutic coverage to ventricular
system.
MRI for target
g definition,, SPGR ( spoiled
p
gradient recalled acquisition) sequences 1 mm
slices
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43
RADIOSURGERY
Most patients tolerate 15 Gy to brainstem
surface at the edge of the lesion.
Dose limitation not required because of
proximity of deep veins/ corpus callosum.
Follow
F ll up radiology
di l
: Germinoma
G
i
(1
month), for benign histologies ( 36 months).
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44
SURGICAL ANATOMY
Most tumors arise from or attached to the
undersurface of velum interpositum.
Tumors rarely extend above velum
velum.
Blood supply comes from within velum mainly
f
from
MP
P.ch
h&LP
P.ch
h with
i h anastomoses to
pericallosal & quadrigeminal artery.
Most tumors are centered at pineal
gland, some extend to For. Monro.
28 February 2011
45
SURGICAL ANATOMY
Mostly
Mostly, ICV
ICV, Galen , Rosenthal & precentral
cerebellar veins surround or cap the periphery
of these tumors
tumors.
Rarely, ICV are ventral to tumor.
Most
M tumors are not highly
hi hl vascular
l except
Pineoblastomas
Hemangioblastomas
Hemangiopericytomas (Angioblastic meningioma)
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46
MANAGEMENT OF HYDROCEPHALUS
If complete tumor removal is anticipated: EVD
at surgery: removal on POD:2/3
Conversion to VP shunt
ETV preferred :
Gradual reduction of ICP
Eliminates potential complications
Infection
Overshunting
Peritoneal seeding by malignant cells
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47
OPERATIVE: BIOPSY
For adjuvant therapy

Metastatic workup
Prognosis & long term follow up
up.
OPEN BIOPSY
More extensive tissue sampling in view of
heterogeneity & mixed cell population
Debulking: favorable response to adjuvant therapy
Risk of postop. bleeding in incompletely resected
tumor bed.
28 February 2011
48
OPERATIVE: BIOPSY
STEREOTACTIC BIOPSY
Suited for patients with multiple lesions, clinical
conditions that contraindicate open
surgery/general anaesthesia.
Multiple specimens to be obtained.
Side cutting cannula preferred over cup forceps
Hemorrhage: Continuous suction & irrigation x 15
min.
28 February 2011
49
STEREOTACTIC APPROACHES
INDICATIONS
For biopsy to achieve diagnosis
Aspiration of cystic masses
Radiosurgery for treatment :
meningioma pineocytoma,
meningioma,
pineocytoma AVM
As adjuvant management with chemotherapy for
germ cell neoplasms & pinealoblastomas.
pinealoblastomas
28 February 2011
50
STEREOTACTIC APPROACHES
ADVANTAGES
G S
Resection may not be necessary depending on the
histopathology
Biopsy may guide effective nonsurgical therapies
Radiosurgery for small volume pathologies
DISADVANTAGES
SSmallll bi
biopsy volume
l
: Diffi
Difficulty
lt iin di
diagnosis
i
Experienced neuropathology team required
Risk of radiation related damage & deficits
28 February 2011
51
STEREOTACTIC BIOPSY
ANTEROLATEROSUPERIOR APPROACH:
Low precoronal entry point just behind the
hairline & just above the superior temporal line
line.
Needle trajectory: Through the frontal lobe
, underneath the lateral ventricle & lateral &
inferior to ICV.
POSEROLATEROSUPERIOR APPROACH:
Entry point near PO junction, for tumors
extendingg laterallyy or superiorly.
p
y
28 February 2011
52
STEREOTACTIC BIOPSY
28 February 2011
53
STEREOTACTIC BIOPSY
28 February 2011
54
ENDOSCOPY
BIOPSY : In conjunction with ETV
FLEXIBLE ENDOSCOPE : Limited trajectory to
tumor through foramen magnum
RIGID ENDOSCOPE : Low frontal burr hole
LIMITATIONS
Limited tissue sampling
Difficulty in hemostasis
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55
SURGICAL APPROACHES
HISTORICAL PERSPECTIVE
Infratentorial supracerebellar [Horsley, Krause
(1913) Stein (1971)]
(1913),
Parietal transcallosal [ Dandy (1921), Kunicki ]
Posterior transventricular [ Van Wegenen,1931
Wegenen 1931 ]
Parietooccipital with splitting of
tentorium/splenium [Heppner
[Heppner,Poppen
Poppen and
Marino, Glasauer,Jamieson,Lazar & Clark]
28 February 2011
56
SURGICAL APPROACHES
HISTORICAL PERSPECTIVE
Transvelum interpositum [Sano]
Lateral paramedian infratentorial [ Van den
Bergh,1990]
Other approaches [ TRANSCALLOSAL :
transforaminal, interfornicial,
subchoroidal transchoroidal] primarily for
subchoroidal,
ant./mid 3rd ventricle tumors.
28 February 2011
57
SURGICAL APPROACHES
28 February 2011
58
POSITIONING
SITTING
LATERAL : Non dominant hemisphere down for
supratentorial approaches
Standard lateral : Nose rotated 30` towards floor
Prone : As above + head extended + rotated 45`
towards floor.
PRONE: Steep angle of tentorium makes it impractical

for infratentorial approaches.
Concorde : Head rotated 15` away to facilitate occipital
lobe retraction( desirable for patients < 3 yrs with
excessively large ventr. with increased risk of collapse).
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59
INFRATENTORIAL SUPRACEREBELLAR
28 February 2011
60
INDICATIONS
Tumor with major bulk in midline
Tumor ventral to velum interpositum & deep
venous system
ADVANTAGES
Minimal risk to deep veins
No
N normall neurall ti
tissue violated
i l t d enroute
t
Exposure comparable with that of other routes
28 February 2011
61
COMPLICATIONS
Risks of sitting position
Limited upgaze & convergence
Ataxia
Cognitive
C
iti iimpairment
i
t
Akinetic mutism
More frequent
f
in patients h
having preop
deficits, prev. radiation or invasive tumors.
28 February 2011
62
SURGICAL TECHNIQUE
Position: Sitting , prone

prone, concorde
Head flexed: tentorium parallel to floor
Midline
idli iincision
i i : inion
i i
C3
Burr holes:
At sagittal sinus above torcula
At lateral aspect of transverse sinus b/l
Suboccipital Midline above for. magnum
28 February 2011
63
SURGICAL TECHNIQUE
Durotomy: gentle curving incision
Microscope with variable objective/275mm
Bridging
id i & precentrall cerebellar
b ll veins
i
cauterized & divided.
Until arachnoid is opened & cerebellum freed
from brainstem, the trajectory is to aimed at
Vein of Galen to avoid injury to ICV/Rosenthal
V.
28 February 2011
64
SURGICAL TECHNIQUE
Internal debulking/capsule dissection
Most difficult & dangerous part : inferior
portion of tumor adherent to dorsal midbrain
midbrain.
Copious irrigation to remove all clots that can
bl k aqueduct.
block
d
28 February 2011
65
LATERAL PARAMEDIAN
INFRATENTORIAL
INDICATIONS
Biopsy
Small quadrigeminal area tumor
ADVANTAGE
Minimal damage to neural tissues
Useful in steep tentorium
Reduced
R d d risk
i k off air
i embolism
b li
DISADVANTAGES
Narrow space
p
Difficult to reach tumor portion extending to inferoposterior
part of 3rd ventricle
28 February 2011
66
LATERAL PARAMEDIAN
INFRATENTORIAL
POSITION
On the side: usually right side down
Upper part of trunk raised 30`
30
Head flexed with neck stretched & rotated 45`
face down
28 February 2011
67
LATERAL PARAMEDIAN INFRATENT

SURGICAL TECHNIQUE
Sshaped incision behind mastoid
Oval craniectomy close to sigmoid sinus laterally &
transverse sinus superiorly
Durotomy : cruciate
Bridging veins divided, petrosal & precentral
cerebellar veins preserved
preserved.
Tentorial incisura reached, preserving SCA.
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68
SUPRATENTORIAL APPROACH
28 February 2011
69
SUPRATENTORIAL APPROACH
INDICATIONS
Tumors extending superiorly
Tumors involving or destroying the posterior
aspect of corpus callosum
Tumors deflecting the deep venous system
dorsolaterally
Tumors extending laterally to region of trigone
Tumors displacing deep veins in ventral direction (
e.g.. Meningiomas)
28 February 2011
70
OCCIPITAL TRANSTENTORIAL
28 February 2011
71
INDICATIONS
Tumors straddling or lying above the tentorial notch
Vascular lesions : varices of vein of Galen, AVM, P3/4
PCA aneurysms.
ADVANTAGES
Excellent
E ll t view
i b
both
th above
b
&b
below
l th
the notch
t h
DISADVANTAGES
Damage to occ
occ. Lobe: visual field defects
defects.
Damage to splenium
Difficult to access subtentorial C/L portion of tumor
28 February 2011
72
Surgical technique
Position: semiprone with nondominant side down
Incision: U
Ushape
shape
Craniotomy : 6 burr holes : 2 on left, 2 on right of
sag Sinus ,1
sag.
1 just rostral to trans
trans. Sinus & 1
parietal.
Durotomy: TT shape & reflected along sinuses
Retractor on inferior surface of occipital lobe
28 February 2011
73
Falx retracted medially
Ventricular drain placed in occipital horn
Tentorium
i
cut 11.5 cm from
f
& parallel
ll l to
straight sinus.
Quadrigeminal cistern opened , CSF drained
Veins visualized : Galen vein right
g
RosenthalICVprecentral cerebellar .
28 February 2011
74
Cleavage plane found in small tumor
Debulking in large tumor
For hypervascular
h
l tumor: feeding
f di arteries
i
identified & coagulated prior to debulking .
To avoid venous injury, total resection is not
necessary & should not be attempted.
Immaculate hemostasis, watertight dura
closure.
28 February 2011
75
28 February 2011
76
28 February 2011
77
PARIETAL INTERHEM. TRANSCALLOSAL
28 February 2011
78
ANTERIOR TRANSCALLOSAL
28 February 2011
79
TRANS VELUM
VELUM INTERPOSITUM
INDICATIONS
Huge tumors in pineal region/posterior 3rd ventricle
Tumors extendingg anterior to adhesio interthalamica
ADVANTAGES
Tumors extending into lateral ventricular can also be
managed
DISADVANTAGES
Damage to anterior corpus callosum
Damage to fornix
28 February 2011
80
TRANS VELUM
VELUM INTERPOSITUM
Surgical technique
Position : Supine with head elevation 20` in pin
Coronal/Quadrangular skin flap on nondominant
side
Quadrangular bone flap , extending to midline &
anterior to coronal suture
Right frontal lobe retracted
retracted, corpus callosum
exposed , split 34 cm to enter pars centralis
28 February 2011
81
TRANS VELUM
VELUM INTERPOSITUM
Velum interpositum ( choroid plexus + tela
choroidea + ICV ) cut just lateral to tenia
fornicis & medial to choroid plexus of lateral
ventricle
B/L fornices & IJV retracted medially to
explore tumor b/w these structures & right
thalamus.
thalamus
28 February 2011
82
COMBINED SUPRAINFRATENT.
TRANSSINUS
Indications
Large tumors > 4.5 cm
Tumor arising from tentorium or extending above
& below
Tumor well below plane of cerebellar retraction (
2cm below sup. Surface of cerebellum)
Very vascular tumors
Tumors encasing imp. Venous structures.
28 February 2011
83
TRANSSINUS
Cerebral angiogram is mandatory to look for
venous anatomy, size & communication b/w
transverse sinus & deep venous system
system.
VP Shunt 24 weeks prior to planned surgery
Somatosensory
S
evoked
k d potentials
i l ffrom UL &
LL B/L & BAER monitoring during surgery
Position : Semiprone with proposed trans.
Sinus section placed inferiorly.
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84
TRANSSINUS
Ushape
U shape incision
Burr holes inferior to trans. Sinus (4) & just
above for.
for Magnum (2) suboccipital
craniotomy
Trans.
T
Si
Sinus separated
d & occipital
i i l craniotomy
i
performed on one side followed by other.
Suboccipital dura opened in transverse
fashion inferior to trans. Sinus
28 February 2011
85
TRANSSINUS
28 February 2011
86
TRANSSINUS
Occipital dura is then opened parallel to
sinuses
20 G butterfly needle inserted in trans. Sinus
just lateral to torcula & medial to temporary
clip placed for test occlusion x 5 min.
Nondominant trans. Sinus can be safely
sectioned if : venous pressure > 5 mm Hg, no
brain swelling, no change in evoked potentials.
Tentorium is then cut parallel to straight sinus.
28 February 2011
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TRANSSINUS
28 February 2011
88
TRANSSINUS
Deep vein injury : repair with 70
7 0 prolene/8
prolene/80
0
nylon
Trans.
Trans Sinus can be reconstructed with a short
vein graft interposed with 60 prolene.
Not
N necessary to suture tentorium
i
Dural graft to allow watertight closure &
expansion of posterior fossa.
p bone kept
p out.
If brain swellingg + : suboccipital
28 February 2011
89
TRANSSINUS
Postoperative Care
Look for respiratory abn in tumors compressing
brainstem
CT on POD1 to l/f pneumocephalus/clots
Nystagmus/ataxia/oscillopsia
Ventricular drain x 34 days 20 cm above head x
24 hrs
hrs test clamping x 24 hrs with ICP
monitoringCT to l/f ventricle size.
Vision abnormalities
28 February 2011
90
COMPLICATIONS OF SURGERY
Postop.
ostop. Hemorrhage/apoplexy
e o age/apop e y
Pupillary abn., accomodation abn., ocular
palsies, upward
p
p
ggaze p
paresis, ataxia, impaired
p
consciousness, shunt malfunction, ETV blockage.
Sitting position: air
embolism, hypotension, cortical
collapse, subdural hygroma
Parietal
P i t l approaches:
h sensory/stereognostic
/t
ti
deficits.
Occipital : Visual field defects
28 February 2011
91
THANK YOU !!
28 February 2011
92

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PINEAL REGION TUMORS

DIAGNOSIS & SURGICAL